OBJECTIVE  To determine the overall survival (OS) and prognostic factors influencing outcomes in children and adolescents with malignant extracranial germ cell tumours (MEGCTs) in preparation for the development of a harmonised national treatment protocol.METHODS A retrospective folder review was undertaken at nine South African paediatric oncology units to document patient profiles, tumour and treatment-related data and outcomes for all children with biopsy proven MEGCTs from birth up to and including 16 years of age. RESULTS Between 1 January 2000 and 31 December 2015, 218 patients were diagnosed with MEGCTs. Female sex (HR 0.284 p=0.037) and higher socio-economic status (SES) (HR 0.071; p=0.039) were associated with a significantly lower risk of death. Advanced clinical stage at diagnosis significantly affected 5-year OS: stage I -96%; stage II - 94.3%; stage III -75.5%; (p=0.017) and stage IV (60.1%; p<0.001). There was a significant association between earlier stage at presentation and higher SES (p=0.03). Patients with a serum AFP level of more than 33,000 ng/ml at diagnosis had significantly poorer outcomes (p=0.002). The use of chemotherapy significantly improved survival, irrespective of the regimen used (p<0.001). CONCLUSIONS The cohort demonstrated a 5-year OS of 80.3% with an EFS of 75.3%. Stage, the use of chemotherapy and an elevated serum AFP level of more than 33,000ng/ml were independently predictive of outcome. The relationship between SES and outcome is important as the implementation of the new national protocol hopes to standardise care across the socio-economic divide. 

Background and objectives: Children with Hodgkin lymphoma (HL) and Human Immunodeficiency Virus (HIV) have low 5 year overall survival rates in South Africa, home to the world’s largest HIV population. We aimed to explore factors impacting on survival in children with HIV and HL by comparing those with and without HIV, and to determine long term survival. Methods: A retrospective study was conducted to determine survival rates and prognostic factors in South African children and adolescents with HL. Univariate risk factor analysis was performed to analyse prognostic factors. Results: Between January 2000 and December 2010, 271 children and adolescents with HL were eligible for analysis. Compared to the 242 HIV-uninfected patients, the 29 HIV-infected patients were younger (median 7.2 vs 10.0 years, p=0.052) and more likely to present with Stage IV disease (p=0.000) The 5-year overall survival rate (OS) of HIV-infected patients of 49% versus 84% for HIV-uninfected patients (p=0.001) appeared to be associated with hypoalbuminaemia (<20g/dL) and a CD4 percentage of <15%. Causes of death in the HIV-infected group included disease progression (6/14), infection (4/14), unknown (3/14) and second malignancy (1/14). The 10-year OS was 45% in the HIV-infected cohort and 79% in the HIV-uninfected cohort (p=0.000). Conclusion: HIV-infected children with HL experience increased mortality due to opportunistic and nosocomial infections. Particular attention should be paid to children and adolescents with hypoalbuminaemia and low CD4 percentages at diagnosis to lower treatment-related mortality. Aggressive supportive care of children with HIV and HL may improve survival.